A giant step
It has been mentioned in the literature that another hormonal abnormality can cause CVG: acromegaly. This disorder is caused by abnormal growth in the brain’s pituitary gland, a tumor that produces excess growth hormone. As a medical student, Mathis had learned to recognize the disorder by searching for traits of the most famous patient of his life with acromegaly, André René Roussimoff, best known by the name he took first in his wrestling career and later as an actor: André The Giant. And he was a giant: 7 feet 4 tall and over 500 pounds. His face, well known to many of us for his role as Fezzik, the friendly giant in Rob Reiner’s classic film “The Princess Bride,” serves as a template for the effects of acromegaly. The excess growth hormone causes enlargement of the soft tissues of the face, including the ears, nose, and tongue, and the soft tissues of the hands and feet. If left untreated, this type of tumor leads to premature death.
Mathis looked at the patient – not just his skin, but the whole person. He was a tall man, over six feet tall and weighing over 250 pounds. He had protruding ears and a large nose. Even his tongue was big. Acromegaly was quite possible. He asked the patient if he had noticed any changes in the size of his feet or hands in recent years. He definitely had, the man replied promptly. A few years ago, he and his wife replaced the wedding rings they bought each other when they got married and didn’t have a lot of money, and he needed a larger ring. And his feet grew too. He used to wear a size 12 but now he needed a size 14. He suspected it was because he was getting bigger. He had gained 75 pounds since their wedding day.
The dermatologist explained his thinking. He had never made a diagnosis of acromegaly and quickly looked up how to do it. The first step was to check the level of one of the growth factors that are normally stimulated by this type of brain tumor. If this were high, the patient would need imaging of their brain to look for a tiny tumor on the pituitary gland. It took days for the blood test to come back. It was abnormal. And the CT scan showed a lima bean-sized growth on his pituitary gland. Mathis referred the patient to a neurosurgeon who removed the tumor. He did not see the patient again until the following year. In the meantime, the patient reported, his fingers and nose had become smaller and his gratitude for the young doctor had grown. Mathis was delighted with his diagnosis. “I’m curious,” he told me recently, and it was one of his most reliable and valuable tools as a doctor. And he’s not ashamed to have to look things up.
Two years have passed since the patient’s surgery, and he and his wife watched with interest as the man’s former face emerged. The lumpy scalp is unchanged, but his wife was especially pleased to see her husband’s old nose again. He’s starting to lose his hair – maybe it took the excess growth hormone to stay – but his wife told me she thought it was a low cost.
Lisa Sanders, MD is a contributing writer for the magazine. Her latest book is Diagnosis: Solving the Most Baffling Medical Mysteries. If you would like to share a resolved case with others, write to her at [email protected]